Son enfermedades autosómicas recesivas caracterizadas por cúmulos de pigmentos lipídicos, autofluorescentes e insolubles, en los lisosomas de las células ganglionares.
Se diferencian las siguientes formas:
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Las tres primeras formas asocian distrofia retiniana severa con grave deterioro neurológico. Las formas adulta y atípica y adulta conservan una visión normal.
- Infantil.
Aparecen retrasos del desarrollo mental hacia los 12-18 meses de vida y complicaciones visuales en algunos casos, a partir de los 12 meses pero siempre alrededor de los 18 meses. A los 2 años o 2 años y medio, ninguno puede percibir ya la luz, no hay reacciones pupilares y los movimientos del ojo carecen de coordinación. A los 3 años, estos niños carecen de coordinación y mueren a la edad media de 6 años y medio.
El fondo de ojo puede ser normal y la distrofia retiniana sólo se diagnostica tras la realización del ERG. - Infantil tardía.
El desarrollo psicomotor del niño es normal hasta los 2-4 años de edad; después aparece la ataxia, se interrumpe el desarrollo, se manifiestan problemas de conducta y, concomitantemente, se sufre p&ecute;rdida de la visión. La enfermedad pregresa hasta la muerte del paciente, entre 1 y 4 años después del iniocio de la sintomatología.
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